Red flags
- Sudden sensorineural hearing loss (SSNHL) - >=30 dB across 3 contiguous frequencies developing over <=72 h
- *An ENT emergency. Steroid within 2 weeks, audiogram within 14 days, and it is a "treat before you image" problem*
- Unilateral or asymmetric sensorineural loss - vestibular schwannoma until MRI says otherwise
- Unilateral hearing loss + facial numbness, ataxia or facial weakness = cerebellopontine angle lesion
- Unilateral middle ear effusion in an adult - *nasopharyngeal carcinoma until proven otherwise*, especially in patients of Southeast Asian ancestry
- Hearing loss + vertigo + acute neurological signs = AICA territory infarct (labyrinthine artery arises from AICA - a "peripheral" presentation of a stroke)
- Pain, discharge, fever, facial palsy, granulation in the canal = necrotising (malignant) otitis externa - diabetic or immunosuppressed, Pseudomonas
- Hearing loss with headache, cranial neuropathies or systemic features - meningitis, sarcoid, GPA, syphilis, leptomeningeal malignancy
- Ototoxic exposure - aminoglycosides, cisplatin, high-dose loop diuretics, high-dose salicylates
- Sudden loss after barotrauma, straining or head injury -> perilymph fistula
Differential by mechanism2 exam ›
Step 1 - conductive or sensorineural?
| Conductive | Sensorineural |
|---|---|
| Wax impaction | Presbyacusis (age-related, bilateral, high-frequency, symmetric) |
| Otitis media / middle ear effusion | Noise exposure (bilateral, 4 kHz notch) |
| Otosclerosis (F, 20-40, family history, worse in pregnancy, low-frequency loss, Carhart notch, paradoxical improvement in noise) | Ototoxicity - aminoglycosides, cisplatin, high-dose aspirin, loop diuretics, quinine, alcohol |
| Cholesteatoma or another destructive lesion (painless foul discharge, attic retraction) | Meniere disease (fluctuating LOW-frequency loss + vertigo + tinnitus + aural fullness) |
| Paget disease of the temporal bone (mixed loss) | Vestibular schwannoma (unilateral, progressive, poor speech discrimination) |
| Tympanic membrane perforation, ossicular disruption | Petrous temporal bone fracture; internal auditory artery/labyrinthine infarction |
| Canal atresia, foreign body, exostoses | Congenital or acquired rubella and syphilis |
- Unilateral SNHL - vestibular schwannoma, petrous temporal bone fracture, labyrinthine/internal auditory artery vascular disease, sudden idiopathic SSNHL, Meniere, mumps, trauma
- Bilateral SNHL - noise exposure, age-related degeneration, ototoxicity (aspirin, gentamicin, alcohol), congenital or acquired rubella or syphilis, Meniere disease
Step 2 - by pattern on the audiogram
- High-frequency downsloping - presbyacusis, ototoxicity
- 4 kHz notch - noise-induced
- Low-frequency - Meniere, otosclerosis (early)
- Flat - sudden SSNHL, autoimmune inner ear disease
- Asymmetric or poor speech discrimination out of proportion to the pure tone average - retrocochlear
Systemic causes not to forget
- Autoimmune inner ear disease, Cogan syndrome (interstitial keratitis + audiovestibular loss), GPA, SLE, sarcoidosis
- Syphilis and HIV; Lyme disease
- Mitochondrial (m.1555A>G) - catastrophic aminoglycoside sensitivity; ask about maternal family history before prescribing gentamicin
- Hypothyroidism, renal failure, Alport syndrome, Pendred syndrome, neurofibromatosis type 2 (bilateral vestibular schwannomas)
Focused history
- Onset: sudden (<72 h) vs progressive over months vs fluctuating
- Unilateral or bilateral - unilateral is the one that gets an MRI
- Associated: tinnitus, vertigo, aural fullness, otalgia, discharge, facial weakness, headache
- Speech discrimination - "I can hear you but I can't understand you", trouble in restaurants
- Noise exposure - occupation, shooting, music, machinery; hearing protection use
- Drugs - aminoglycosides, cisplatin/carboplatin, loop diuretics, salicylates, quinine, vancomycin, macrolides; cumulative dose and timing
- Trauma, barotrauma, diving, straining; head injury
- Family history - otosclerosis, hereditary deafness, NF2, mitochondrial (maternal line)
- Perinatal - rubella, CMV, prematurity, kernicterus, meningitis in childhood
- Systemic: eye symptoms, sinus disease, rash, arthralgia, renal disease, sexual health history
- Functional impact - social withdrawal, work, telephone use, safety; and screen for depression and cognitive decline
Focused examination2 exam ›
Otoscopy first - the commonest cause is wax
- Canal (wax, foreign body, otitis externa, exostoses, granulation)
- Tympanic membrane - perforation, effusion (fluid level, retraction), attic retraction or crust = cholesteatoma, tympanosclerosis
- In an adult with a unilateral effusion, examine the postnasal space (nasopharyngeal carcinoma)
Tuning fork tests - 512 Hz
| Test | Sensorineural (nerve) deafness | Conductive deafness |
|---|---|---|
| Rinne | "Rinne positive" - air conduction still LOUDER than bone conduction (both are reduced roughly equally) | "Rinne negative" - bone conduction LOUDER than air, or air conduction inaudible at the meatus |
| Weber (forehead midline) | Lateralises to the NORMAL ear | Lateralises to the ABNORMAL (affected) ear |
- **False negative Rinne: in severe unilateral SNHL, bone conduction crosses to the good cochlea and appears "negative" - use masking, or trust the Weber**
Then
- Cranial nerves - especially V (corneal reflex), VII, and cerebellar signs (cerebellopontine angle)
- Fistula test, head impulse test, nystagmus, Dix-Hallpike if vertigo
- Neck and postnasal space; cervical lymph nodes
- Whispered voice test at 60 cm with the other ear masked - a reasonable bedside screen
- General: blue sclerae/skeletal features (osteogenesis imperfecta), eyes (Cogan, Usher), skin, joints, urinalysis (Alport, GPA)
Investigation strategy
Everyone
- Pure tone audiometry with air and bone conduction, plus speech discrimination - the single essential test
- In suspected SSNHL, within 14 days of onset
- Tympanometry - type B (effusion), type C (eustachian dysfunction), type As (otosclerosis, shallow), absent stapedial reflexes in otosclerosis
Directed
| Suspicion | Test |
|---|---|
| Unilateral/asymmetric SNHL, or SSNHL | MRI internal auditory meati with gadolinium - the gold standard for vestibular schwannoma. ABR where MRI is unavailable or contraindicated |
| Cholesteatoma, chronic otitis media, trauma | High-resolution CT temporal bone |
| Meniere | Audiometry during an attack (low-frequency loss), electrocochleography, MRI to exclude a structural cause |
| Systemic/autoimmune | ESR/CRP, ANA/ENA, ANCA, RF, complement, syphilis serology, HIV, TFT, UEC, FBE |
| Vasculitis/sarcoid | ACE, CXR/CT chest, urinalysis with microscopy |
| Suspected genetic | GJB2 (connexin-26), m.1555A>G before aminoglycosides, NF2 gene testing if bilateral schwannomas |
| Children/infants | Otoacoustic emissions, ABR, congenital CMV PCR from the newborn screening card |
- Blood tests are low yield in typical presbyacusis - do not order a vasculitic screen for a symmetric high-frequency loss in an 80-year-old
Management
A. Sudden sensorineural hearing loss - the emergency
- Confirm with audiometry, do not wait for it to treat
- Oral corticosteroid - prednisolone 1 mg/kg/day (max ~60-80 mg) for 7-14 days then taper - started within 2 weeks of onset
- The earlier the better; benefit falls off sharply after 2 weeks and is negligible after 6 weeks
- Intratympanic dexamethasone - as salvage after incomplete recovery, or as primary therapy where systemic steroid is contraindicated (diabetes, immunosuppression); increasingly used concurrently in severe loss
- MRI IAM (or ABR) in every case - up to 3% harbour a vestibular schwannoma
- *Do not offer antivirals, thrombolytics, vasodilators or antioxidants - no evidence*
- Hyperbaric oxygen within 2 weeks may be offered as an adjunct where available
- Counsel: ~2/3 recover at least partly, most within 2 weeks; poor prognosis with severe loss, vertigo, older age, or delayed treatment
B. Conductive causes
- Wax - softening drops then irrigation or microsuction (not irrigation if perforation or grommets)
- Otitis media with effusion - watchful waiting; grommets if persistent; in adults, always examine the postnasal space
- Chronic suppurative otitis media - aural toilet, topical antibiotic; ENT referral
- Cholesteatoma - surgical: it is locally destructive and will not resolve (facial nerve, labyrinth, intracranial extension)
- Otosclerosis - stapedotomy (excellent results) or hearing aid; fluoride and bisphosphonates are not established
- Perforation - keep dry, myringoplasty if persistent
C. Sensorineural causes
- Presbyacusis - hearing aids, early; communication strategies, assistive listening devices
- *Untreated hearing loss is the largest single potentially modifiable risk factor for dementia in the Lancet Commission - this is the argument that gets people to accept aids*
- Noise-induced - prevention only (hearing protection, noise limits); it does not recover; workers compensation and occupational reporting
- Ototoxicity - stop or substitute; monitor audiometry during aminoglycoside and cisplatin courses; check m.1555A>G if a family history
- Meniere disease - salt restriction, betahistine, thiazide; intratympanic steroid or gentamicin, endolymphatic sac surgery in refractory cases
- Autoimmune inner ear disease - high-dose corticosteroid, then methotrexate/rituximab; responds rapidly to steroid - a therapeutic trial is diagnostic
- Vestibular schwannoma - observation with serial MRI for small tumours, radiosurgery, or microsurgery; managed in a skull base MDT
D. Rehabilitation - always
- Hearing aids - refer early; Hearing Services Program funding for pensioners and veterans in Australia
- Cochlear implant for severe-profound bilateral loss with poor aided speech discrimination - adults do well, and referral is often left far too late
- Bone-anchored hearing devices for conductive/mixed loss or single-sided deafness
- Speech pathology and audiological rehabilitation; captioning, telecoil, assistive devices
- Tinnitus - CBT and sound therapy; treating the hearing loss usually improves the tinnitus
- Address social isolation, depression and cognitive decline; workplace and driving/safety implications
Traps
- Rinne "positive" is the NORMAL result - and it is also the result in sensorineural loss. The Weber is what separates them
- *A false negative Rinne occurs in severe unilateral SNHL* - the sound crosses to the opposite cochlea by bone conduction
- Sudden sensorineural hearing loss is treated as an emergency, but is routinely triaged as "ear wax" - if the ear looks normal and hearing dropped over hours, it is SSNHL until an audiogram says otherwise
- A unilateral middle ear effusion in an adult is nasopharyngeal carcinoma until the postnasal space is examined
- Sudden hearing loss with vertigo can be an AICA infarct - check for other posterior circulation signs, and do not be reassured by a "peripheral" pattern
- Vestibular schwannoma classically causes speech discrimination far worse than the pure tone average predicts
- Bilateral vestibular schwannomas = neurofibromatosis type 2 - and the patient's children need screening
- Aminoglycoside ototoxicity is often bilateral, permanent, and delayed - and m.1555A>G carriers lose their hearing after a single dose
- Cholesteatoma is painless with a foul discharge - the absence of pain is what makes it dangerous
- Do not irrigate an ear with a perforation or grommet
- Hearing loss is a major and under-treated risk factor for dementia, delirium and falls - "he's just getting old" is not a plan
Talk track
1. First split
- "I'd start with otoscopy and tuning forks to separate conductive from sensorineural loss, because that halves the differential immediately - Weber lateralises to the affected ear in conductive loss and away from it in sensorineural loss."
2. The emergency
- "The one I would not miss is sudden sensorineural hearing loss - 30 decibels across three contiguous frequencies within 72 hours. That gets high-dose oral corticosteroid started within two weeks, an urgent audiogram, and an MRI of the internal auditory meati."
3. The tumour
- "Any unilateral or asymmetric sensorineural loss gets an MRI for vestibular schwannoma, and I'd be alerted by speech discrimination that is worse than the audiogram predicts."
4. Pattern recognition
- "Then I'd use the audiogram pattern - a 4 kHz notch for noise, a downsloping high-frequency loss for presbyacusis, and a fluctuating low-frequency loss with vertigo and aural fullness for Meniere disease."
5. Cause-directed treatment
- "Wax and effusion are treatable, cholesteatoma is surgical, and ototoxic drugs need to be stopped or substituted with audiometric monitoring."
6. Close on function
- "And I'd finish on rehabilitation - early hearing aids, cochlear implant referral if aided speech discrimination is poor, and an explicit conversation about isolation and cognitive decline, because untreated hearing loss is the largest modifiable risk factor for dementia."
8 of 8 sections written · drafted 2026-09-04